Escolar Documentos
Profissional Documentos
Cultura Documentos
65
Open Access
Keywords: Optic neuritis, differential diagnosis of optic neuritis, multiple sclerosis, neuromyelitis optica.
INTRODUCTION
The term optic neuritis (ON) refers to inflammation of
the optic nerve due to many causes, indicated by sub-acute
unilateral painful visual loss mostly in a young healthy
female and by excluding glaucoma, ON is the most common
optic neuropathy in persons under 50 years coming to
general ophthalmic practice. It is the earliest clinical
symptom in about 20% of cases of MS [1-5].
The gold standard treatment for ON is based on the Optic
Neuritis Treatment Trial (ONTT) which was undertaken in
order to determine the efficacy of corticosteroids and to
permit long-term analyses [1,6,7]. In the ONTT, 15 clinical
centers in the United States registered 457 patients between
July 1, 1988 and June 30, 1991 with the following criteria:
the presence of acute unilateral optic neuritis with visual
symptoms for 8 days or less, age between18 and 45 years, no
previous history of ON in the affected eye, no evidence of
systemic disease other than MS that might be associated with
the ON, and no previous treatment with corticosteroids for
MS or ON [1,6,7]. This article reviews available studies
published in English and Spanish regarding optic neuritis, its
differential diagnosis and management.
AETIOLOGY
ON is mainly idiopathic in nature; though, it could be related to demyelinating lesions (e.g. MS [4,8-10] neuromyelitis
*Address correspondence to this author at A7/503, 10 Elite Society, Kate
Puram Chowk, New Sangvi, Pimple Gurav, Pune 411027, Maharashtra,
India; Tel: +91-9604304202;
E-mails: farid_bagherkashi@yahoo.com, circleofunity9@yahoo.com
1874-3641/12
Table 1.
Demylinating
lesions
Multiple Sclerosis (MS) [4,8-10], Neuromyelitis optica [4,8,9], Shilders disease, Encephalitis periaxialis concentrica [4] (out of
which MS is the most common cause [4])
Autoimmune
Disease
Sarcoidosis [1,4,9], systemic lupus erythematosus (SLE) [4,9], Sjgrens syndrome (SS), Behchets disease [9]
Infectious/parainfectious
Herpes zoster [9], lyme disease [1,4,9], syphilis [1,4,9,11], tuberculosis, dengue [11,13], mumps, varicella zoster [4,11],
toxoplasmosis [4,9,11], measles [4,11,12], leptospirosis, chickungunya, west nile [11], adenovirus, brucellosis, coxsackievirus, cat
scratch disease, -hemolytic streptococcal infection, meningococcal infection, typhoid fever, whipples disease [4]
Inflammatory/post
vaccination
sinusitis [1], vaccinations against tuberculosis, hepatitis B, rabies, tetanus, meningitis, anthrax, measles, rubella & influenza [12]
Table 2.
Table 3.
Severe visual loss (no light perception) which progress for >2 weeks
from onset [1,2,4,9]
o
o
o
o
o
o
DIFFERENTIAL DIAGNOSIS
PREVALENCE
Patients with acute demyelinating ON are typically
healthy young adults. Female preponderance in observed,
with a ratio of approximately 3:1. For reasons still unclear,
the incidence of MS associated with ON is highest in people
living at higher latitudes (e.g.in northern USA, northern and
western Europe; New Zealand and southern Australasia) and
reduce significantly closer to the equator. Studies have
reported a correlation between reduction in vitamin D (25hydroxyvitamin D) level and increased risk of
developing/relapsing MS. Therefore lower intensity and
lesser exposure to sunlight at high latitude may be an
explanation for epidemiological variations of MS. The
Table 4.
67
Neuromyelitis Optica
Multiple Sclerosis
Usually [8,9]
Rarely [8]
Usually [8,22]
Transverse myelitis
Rarely [8]
Rarely [8]
Usually [8]
Oligoclonal bands
Rarely [2,8,9]
Frequently [8,22]
Protein contents
DMDs
Effective [8,9,23]
Immunosuppressive (corticosteroids)
CSF Analysis
Treatment
Table 5.
Absolute criteria:
Optic Neuritis
Acute myelitis
Supportive criteria:
Brain MRI not meeting diagnostic criteria for MS
Spinal cord MRI that has T2 signal abnormalities extending over
three or more vertebral segments
NMO-IgG seropositive status
69
TREATMENT
The goals of many of the existing and emerging
treatments including steroid and immuno-modulatory
therapy are reduction in the number and severity of attacks,
and prevention of axonal loss and subsequent disability in
both ON and MS [8].
Recovery of visual functions in ON is observed
spontaneously within 2-3 weeks in more than 80% of
patients without treatment. Vision stabilizes over months or
continues to improve up to 1 year, although long-term
defects in visual functions is possible [2,5,9,31,32].
According to the Optic Neuritis Treatment Trial (ONTT)
protocol, each patient was randomly assigned to receive one
of the following three regimens within 8 days after the onset
of symptoms: (1) oral prednisolone, 1 mg/kg per day for 14
days (prednisone group); (2) intravenous methylprednisolone
(IVMP) sodium succinate, 250 mg every 6 hours for 3 days,
followed by oral prednisone, 1 mg/kg per day for 11 days
(intravenous group); or (3) oral placebo for 14 days (placebo
group). Regimens for oral and intravenous prednisolone
groups were followed by a short tapering off with oral
dosages consisting of 20 mg of prednisolone on day 15 and
10 mg on days 16 and 18 [1,2,4,6,10,35].
ONTT and other studies showed that the high-dose
intravenous corticosteroids were effective in improving
short-term visual recovery particularly for visual fields and
contrast sensitivity as compared to oral prednisolone and
placebo, but the difference in the rate of recovery subsided
within 1 month and there was no statistically significant
benefit in long-term (1year) outcome among the three
groups. At 6 months, there was still a small statistically
significant benefit for the intravenous regimen in contrast
sensitivity, visual field, and color vision but not in visual
acuity [1-4,6,9,10,31,32,35].
Based on the ONTT findings, intravenous steroids
treatment is recommended when 3 or more signal
abnormalities are present on MRI and reduces risk of
developing MS by 2 years. It is also recommended in
patients in whom there is a need for faster recovery of visual
deficits (i.e., uniocular patients, employment demands,
bilateral involvement and patients desiring intervention)
[2,4,10,32].
Fig. (1). (A, B) MRI of brain and orbits in pateint with acute
demyelinating optic neuritis. Reprinted by permission from [N Engl
J Med 2006; 354: 1273-80. Copyright 2006 Massachusetts
Medical Society].
Immunnomodulatory Therapy
RISK OF RECURRENCE OF ON
Optic neuritis can occur as a monophasic or recurrent
disease, either in the same or the contralateral eye especially
in patients who develop MS thereafter. The ONTT reported
28% and 35% of patients recurred ON within 5 to 10 years
respectively [2,14]. At the 5 year follow-up, the relapse of
ON was 19% for the affected eye, 17% for the fellow eye
and 30% for either eye [1].
Treatment with oral prednisone alone in standard doses
increased the relapse rate of ON and therefore is not
recommended in acute typical ON (Table 6). Higher doses of
oral corticosteroids have shown the same recurrence rate
compared to placebo [1,3,6,8,10,32,35].
Table 6.
71
50%
45%
40%
35%
30%
25%
20%
15%
10%
5%
0%
44%
41%
30%
29%
311%
%
25% 25%
13%
16%
2 years
Oral prednisolone
5 years
IVMP
10 years
Placebo
[1]
[2]
[3]
[4]
[5]
[6]
[7]
[8]
[9]
[10]
[11]
[12]
[13]
[14]
[15]
[16]
[17]
[18]
[19]
[20]
[21]
[22]
[23]
[24]
[25]
[26]
[27]
[28]
[29]
[30]
[31]
[32]
[33]
[34]
[35]